The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review

We present eight cases of the homozygous MCPggaac haplotype, which is considered to increase the likelihood and severity of atypical hemolytic uremic syndrome (aHUS), especially in combination with additional risk aHUS mutations. Complement blockade (CBT) was applied at a median age of 92 months (IQ...
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Gespeichert in:

Autor*in:	Daniel Turudic [verfasserIn] Danka Pokrajac [verfasserIn] Velibor Tasic [verfasserIn] Dino Kasumovic [verfasserIn] Zoltan Prohaszka [verfasserIn] Danko Milosevic [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2023

Schlagwörter:	aHUS complement blockade MCPggaac children Southeastern Europe

Übergeordnetes Werk:	In: International Journal of Molecular Sciences - MDPI AG, 2003, 24(2023), 17, p 13041
Übergeordnetes Werk:	volume:24 ; year:2023 ; number:17, p 13041

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc Journal toc

DOI / URN:	10.3390/ijms241713041

Katalog-ID:	DOAJ09351512X

Internformat


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allfields	10.3390/ijms241713041 doi (DE-627)DOAJ09351512X (DE-599)DOAJf696f0be545d46b2ac1fb9e182029ed8 DE-627 ger DE-627 rakwb eng QH301-705.5 QD1-999 Daniel Turudic verfasserin aut The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier We present eight cases of the homozygous MCPggaac haplotype, which is considered to increase the likelihood and severity of atypical hemolytic uremic syndrome (aHUS), especially in combination with additional risk aHUS mutations. Complement blockade (CBT) was applied at a median age of 92 months (IQR 36–252 months). The median number of relapses before CBT initiation (Eculizumab) was two. Relapses occurred within an average of 22.16 months (median 17.5, minimum 8 months, and maximum 48 months) from the first subsequent onset of the disease (6/8 patients). All cases were treated with PI/PEX, and rarely with renal replacement therapy (RRT). When complement blockade was applied, children had no further disease relapses. Children with MCPggaac haplotype with/without additional gene mutations can achieve remission through renal replacement therapy without an immediate need for complement blockade. If relapse of aHUS occurs soon after disease onset or relapses are repeated frequently, a permanent complement blockade is required. However, the duration of such a blockade remains uncertain. If complement inhibition is not applied within 4–5 relapses, proteinuria and chronic renal failure will eventually occur. aHUS complement blockade MCPggaac children Southeastern Europe Biology (General) Chemistry Danka Pokrajac verfasserin aut Velibor Tasic verfasserin aut Dino Kasumovic verfasserin aut Zoltan Prohaszka verfasserin aut Danko Milosevic verfasserin aut In International Journal of Molecular Sciences MDPI AG, 2003 24(2023), 17, p 13041 (DE-627)316340715 (DE-600)2019364-6 14220067 nnns volume:24 year:2023 number:17, p 13041 https://doi.org/10.3390/ijms241713041 kostenfrei https://doaj.org/article/f696f0be545d46b2ac1fb9e182029ed8 kostenfrei https://www.mdpi.com/1422-0067/24/17/13041 kostenfrei https://doaj.org/toc/1661-6596 Journal toc kostenfrei https://doaj.org/toc/1422-0067 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 24 2023 17, p 13041
spelling	10.3390/ijms241713041 doi (DE-627)DOAJ09351512X (DE-599)DOAJf696f0be545d46b2ac1fb9e182029ed8 DE-627 ger DE-627 rakwb eng QH301-705.5 QD1-999 Daniel Turudic verfasserin aut The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier We present eight cases of the homozygous MCPggaac haplotype, which is considered to increase the likelihood and severity of atypical hemolytic uremic syndrome (aHUS), especially in combination with additional risk aHUS mutations. Complement blockade (CBT) was applied at a median age of 92 months (IQR 36–252 months). The median number of relapses before CBT initiation (Eculizumab) was two. Relapses occurred within an average of 22.16 months (median 17.5, minimum 8 months, and maximum 48 months) from the first subsequent onset of the disease (6/8 patients). All cases were treated with PI/PEX, and rarely with renal replacement therapy (RRT). When complement blockade was applied, children had no further disease relapses. Children with MCPggaac haplotype with/without additional gene mutations can achieve remission through renal replacement therapy without an immediate need for complement blockade. If relapse of aHUS occurs soon after disease onset or relapses are repeated frequently, a permanent complement blockade is required. However, the duration of such a blockade remains uncertain. If complement inhibition is not applied within 4–5 relapses, proteinuria and chronic renal failure will eventually occur. aHUS complement blockade MCPggaac children Southeastern Europe Biology (General) Chemistry Danka Pokrajac verfasserin aut Velibor Tasic verfasserin aut Dino Kasumovic verfasserin aut Zoltan Prohaszka verfasserin aut Danko Milosevic verfasserin aut In International Journal of Molecular Sciences MDPI AG, 2003 24(2023), 17, p 13041 (DE-627)316340715 (DE-600)2019364-6 14220067 nnns volume:24 year:2023 number:17, p 13041 https://doi.org/10.3390/ijms241713041 kostenfrei https://doaj.org/article/f696f0be545d46b2ac1fb9e182029ed8 kostenfrei https://www.mdpi.com/1422-0067/24/17/13041 kostenfrei https://doaj.org/toc/1661-6596 Journal toc kostenfrei https://doaj.org/toc/1422-0067 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 24 2023 17, p 13041
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allfieldsSound	10.3390/ijms241713041 doi (DE-627)DOAJ09351512X (DE-599)DOAJf696f0be545d46b2ac1fb9e182029ed8 DE-627 ger DE-627 rakwb eng QH301-705.5 QD1-999 Daniel Turudic verfasserin aut The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier We present eight cases of the homozygous MCPggaac haplotype, which is considered to increase the likelihood and severity of atypical hemolytic uremic syndrome (aHUS), especially in combination with additional risk aHUS mutations. Complement blockade (CBT) was applied at a median age of 92 months (IQR 36–252 months). The median number of relapses before CBT initiation (Eculizumab) was two. Relapses occurred within an average of 22.16 months (median 17.5, minimum 8 months, and maximum 48 months) from the first subsequent onset of the disease (6/8 patients). All cases were treated with PI/PEX, and rarely with renal replacement therapy (RRT). When complement blockade was applied, children had no further disease relapses. Children with MCPggaac haplotype with/without additional gene mutations can achieve remission through renal replacement therapy without an immediate need for complement blockade. If relapse of aHUS occurs soon after disease onset or relapses are repeated frequently, a permanent complement blockade is required. However, the duration of such a blockade remains uncertain. If complement inhibition is not applied within 4–5 relapses, proteinuria and chronic renal failure will eventually occur. aHUS complement blockade MCPggaac children Southeastern Europe Biology (General) Chemistry Danka Pokrajac verfasserin aut Velibor Tasic verfasserin aut Dino Kasumovic verfasserin aut Zoltan Prohaszka verfasserin aut Danko Milosevic verfasserin aut In International Journal of Molecular Sciences MDPI AG, 2003 24(2023), 17, p 13041 (DE-627)316340715 (DE-600)2019364-6 14220067 nnns volume:24 year:2023 number:17, p 13041 https://doi.org/10.3390/ijms241713041 kostenfrei https://doaj.org/article/f696f0be545d46b2ac1fb9e182029ed8 kostenfrei https://www.mdpi.com/1422-0067/24/17/13041 kostenfrei https://doaj.org/toc/1661-6596 Journal toc kostenfrei https://doaj.org/toc/1422-0067 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 24 2023 17, p 13041
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author	Daniel Turudic
spellingShingle	Daniel Turudic misc QH301-705.5 misc QD1-999 misc aHUS misc complement blockade misc MCPggaac misc children misc Southeastern Europe misc Biology (General) misc Chemistry The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review
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topic_title	QH301-705.5 QD1-999 The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review aHUS complement blockade MCPggaac children Southeastern Europe
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title	The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review
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title_sort	rationale of complement blockade of the mcp<sub<ggaac</sub< haplotype following atypical hemolytic uremic syndrome of three southeastern european countries with a literature review
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title_auth	The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review
abstract	We present eight cases of the homozygous MCPggaac haplotype, which is considered to increase the likelihood and severity of atypical hemolytic uremic syndrome (aHUS), especially in combination with additional risk aHUS mutations. Complement blockade (CBT) was applied at a median age of 92 months (IQR 36–252 months). The median number of relapses before CBT initiation (Eculizumab) was two. Relapses occurred within an average of 22.16 months (median 17.5, minimum 8 months, and maximum 48 months) from the first subsequent onset of the disease (6/8 patients). All cases were treated with PI/PEX, and rarely with renal replacement therapy (RRT). When complement blockade was applied, children had no further disease relapses. Children with MCPggaac haplotype with/without additional gene mutations can achieve remission through renal replacement therapy without an immediate need for complement blockade. If relapse of aHUS occurs soon after disease onset or relapses are repeated frequently, a permanent complement blockade is required. However, the duration of such a blockade remains uncertain. If complement inhibition is not applied within 4–5 relapses, proteinuria and chronic renal failure will eventually occur.
abstractGer	We present eight cases of the homozygous MCPggaac haplotype, which is considered to increase the likelihood and severity of atypical hemolytic uremic syndrome (aHUS), especially in combination with additional risk aHUS mutations. Complement blockade (CBT) was applied at a median age of 92 months (IQR 36–252 months). The median number of relapses before CBT initiation (Eculizumab) was two. Relapses occurred within an average of 22.16 months (median 17.5, minimum 8 months, and maximum 48 months) from the first subsequent onset of the disease (6/8 patients). All cases were treated with PI/PEX, and rarely with renal replacement therapy (RRT). When complement blockade was applied, children had no further disease relapses. Children with MCPggaac haplotype with/without additional gene mutations can achieve remission through renal replacement therapy without an immediate need for complement blockade. If relapse of aHUS occurs soon after disease onset or relapses are repeated frequently, a permanent complement blockade is required. However, the duration of such a blockade remains uncertain. If complement inhibition is not applied within 4–5 relapses, proteinuria and chronic renal failure will eventually occur.
abstract_unstemmed	We present eight cases of the homozygous MCPggaac haplotype, which is considered to increase the likelihood and severity of atypical hemolytic uremic syndrome (aHUS), especially in combination with additional risk aHUS mutations. Complement blockade (CBT) was applied at a median age of 92 months (IQR 36–252 months). The median number of relapses before CBT initiation (Eculizumab) was two. Relapses occurred within an average of 22.16 months (median 17.5, minimum 8 months, and maximum 48 months) from the first subsequent onset of the disease (6/8 patients). All cases were treated with PI/PEX, and rarely with renal replacement therapy (RRT). When complement blockade was applied, children had no further disease relapses. Children with MCPggaac haplotype with/without additional gene mutations can achieve remission through renal replacement therapy without an immediate need for complement blockade. If relapse of aHUS occurs soon after disease onset or relapses are repeated frequently, a permanent complement blockade is required. However, the duration of such a blockade remains uncertain. If complement inhibition is not applied within 4–5 relapses, proteinuria and chronic renal failure will eventually occur.
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title_short	The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review
url	https://doi.org/10.3390/ijms241713041 https://doaj.org/article/f696f0be545d46b2ac1fb9e182029ed8 https://www.mdpi.com/1422-0067/24/17/13041 https://doaj.org/toc/1661-6596 https://doaj.org/toc/1422-0067
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up_date	2024-07-03T17:46:52.698Z
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Complement blockade (CBT) was applied at a median age of 92 months (IQR 36–252 months). The median number of relapses before CBT initiation (Eculizumab) was two. Relapses occurred within an average of 22.16 months (median 17.5, minimum 8 months, and maximum 48 months) from the first subsequent onset of the disease (6/8 patients). All cases were treated with PI/PEX, and rarely with renal replacement therapy (RRT). When complement blockade was applied, children had no further disease relapses. Children with MCPggaac haplotype with/without additional gene mutations can achieve remission through renal replacement therapy without an immediate need for complement blockade. If relapse of aHUS occurs soon after disease onset or relapses are repeated frequently, a permanent complement blockade is required. However, the duration of such a blockade remains uncertain. 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The Rationale of Complement Blockade of the MCP<sub<ggaac</sub< Haplotype following Atypical Hemolytic Uremic Syndrome of Three Southeastern European Countries with a Literature Review

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